- Congenital Vascular Malformations
- NICH (Non-involuting congenital hematoma): rare form of hemangioma that is present at birth, stable over time, and often has a white grey rim
- Stains negative for GLUT-1
- Congenital (infantile) hemangioma:
- Present between 2 weeks and 2 months of life, rapid proliferation during first 9 months, gradual involution until 3.5 years
- have herald patch with rapid growth (most distinguishable factor from vascular malformation),have slow regression, more common in girls and premature infants, can be in parotid gland or liver but nervous system is rare
- Parotid tumor infantile hemangiomas frequently are wrapped in facial nerve and difficult to completely resect –> observe
- Liver is the most common extracutaneous site
- Propranolol for first line hemangioma
- –> observation for small non changing and not in cosmetic areas (laser and steroid (oral) does not work as well)
- Adverse effects of propranolol: lethargy, hypotension, hypoglycemia, bradycardia
- Intramuscular hemangioma: first line is propranolol or steroids –> then excision
- Surgical excision is definitive treatment for lip hemangiomas; propranolol is good for proliferative phase
- Vascular malformation
- Structural abnormalities of the capillary, venous, lymphatic, and arterial systems that grows in proportion to child as opposed to vasoproliferative neoplasms that have increased endothelial cell turnover and proliferate
- AV malformations have bruit or thrill, may have high-output cardiac failure such as in Parkes-Weber syndrome
- Ulceration is associated with increased risk of recurrence
- Treat with embolization and resection
- Lymphatic malformation- aberrant formation of lymphatic vessels slow flow lesion, macrocytic lymphatic malformations treated with sclerotherapy
- Most common on neck and axilla
- Venous malformation- bluish hue, present at birth, don’t resolve over time, symptoms are exacerbated by dependency, treated with sclerotherapy
- Kaposiform hemangioendothelioma: hemangiomas or diffuse hemangiomatosis. malignant vascular tumor which is often associated with kasabach-merritt syndrome. Starts in infancy, can be in trunk and extremities. Skin overlying is deep red-purple, tense, and shiny
- kasabach-merritt phenomenon- bruising, thrombocytopenia, bleeding –> give vincristine or rapamycin
- Treat with chemotherapy and resection (vincrystine)
- Pathologic arterial dilations:
- Pseudoaneuryms: typically result from external or internal insult that causes rupture of endothelium and bleeding (do not involve all layers)
- True aneurysm: endothelial-lined widening of an artery that has all three layers of the vessel lining
- Fusiform in appearance
- Kawasaki disease: true aneurysms (fusiform and dilated appearance), caused by arteriorsclerosis
- Mafucci syndrome: venous malformations and endochondromas (can have malignant chrondroarcomas and intracranial tumors)
- Risk for pathologic fractures
- Sturge Weber: Facial capillary malformations, port wine stain v1/v2, vascular malformation on i/l side, seizure disorders
- Dark purple cobblestoning of cheek is port wine stain
- Klippel Trenaunay: patchy port wine stains, hemi hypertrophy of LE and lymphatic venous malformations (varicosities)
- Associated with limb length discrepancy
- Parkes-Weber: Like KT but with AV malformations
- Risk of high output cardiac failure
- Osler-Weber-Rendu: hereditary hemorrhagic telangectasia, AD and emerge later in life hands, feet, nails etc
- Miscellaneous:
- Maximum defect that can be primarily closed in a scalp is 7cm
